ABSTRACT
The objective of the present study was to increase the frying stability of refined safflower oil (RSO) by blending it with refined olive pomace oil (ROPO) during deep fat frying. For this purpose; RSO, ROPO and their blends were utilized for frying of potato sticks at 180°C for 3 consecutive days. The frying stability of the oils was monitored by analyzing them for their free fatty acids, peroxide values, total polar contents, ultraviolet spectrophotometric indices at 232 and 270 nm, fatty acid profiles, p-anisidine values, α-tocopherol contents and photometric color indices. 3-monochloropropane-1,2-diol (3-MCPD) and glycidyl ester (GE) levels of oils before and after frying were measured as well. The results have shown that thermooxidative degradation products increased as the frying progressed for all oils, however the decomposition rate was found to slow down in blend oils by stabilizing with ROPO. Blending RSO with ROPO decreased linoleic and linolenic; but increased the oleic and palmitic acid percentages of the blends. C18:2/C16:0 ratio was found to decrease by frying for RSO and the blend oils, however ROPO was not affected significantly. 3-MCPD-E levels of the blends increased as the ratio of ROPO increased. Principal component analysis enabled a clear discrimination between oils with different composition throughout the frying process.
ABSTRACT
BACKGROUND: Sitosterolemia is a rare lipid disorder caused by mutations in adenosine triphosphate-binding cassette genes (ABCG) 5 and 8. OBJECTIVE: To evaluate the phenotypic/genotypic features of sitosterolemia in a group of Turkish patients. METHODS: Seven probands with unexplained hematologic abnormalities and their 13 relatives were enrolled. Sterol levels were measured by gas chromatography and genetic studies were performed using Sanger sequencing. Individuals were diagnosed with sitosterolemia if they were found to have frankly elevated sitosterol level >15 µg/mL and/or pathogenic variants of the ABCG5/ABCG8. RESULTS: The seven probands and their six relatives were diagnosed with frank sitosterolemia, and all these patients had hematologic abnormalities. The remaining seven relatives were asymptomatic heterozygous carriers. Three novel variants in the ABCG5 gene (c.161G>A, c.1375C>T, IVS10-1G>T), one novel variant in the ABCG8 gene (c.1762G>C) and one known variant in the ABCG5 gene (c.1336 C>T) were identified. No variant was identified in one case. The mean sitosterol level was significantly higher and mean platelet count was significantly lower in patients with homozygous variants compared to heterozygous variants (p<0.05, for all). Diets low in plant sterols were recommended for 13 symptomatic cases. Four homozygotes received ezetimibe, and their splenomegaly, anemia, and thrombocytopenia completely resolved except one. CONCLUSION: The five pathogenic variants identified in this study indicate the genetic heterogeneity of sitosterolemia in Turkish population. Patients with unexplained hematologic abnormalities (specifically macrothrombocytopenia) should have their sterol level measured as initial testing. Ezetimibe can be a good choice for sitosterolemia.
Subject(s)
ATP Binding Cassette Transporter, Subfamily G, Member 5/genetics , ATP Binding Cassette Transporter, Subfamily G, Member 8/genetics , Hypercholesterolemia/blood , Hypercholesterolemia/genetics , Intestinal Diseases/blood , Intestinal Diseases/genetics , Lipid Metabolism, Inborn Errors/blood , Lipid Metabolism, Inborn Errors/genetics , Lipoproteins/genetics , Mutation , Phytosterols/adverse effects , Sitosterols/blood , Adolescent , Adult , Biomarkers/blood , Child , Child, Preschool , Chromatography, Gas , Female , Genotype , Heterozygote , Humans , Hypercholesterolemia/diagnosis , Intestinal Diseases/diagnosis , Lipid Metabolism, Inborn Errors/diagnosis , Male , Middle Aged , Phenotype , Phytosterols/blood , Phytosterols/genetics , Sequence Analysis, DNA/methods , Turkey , Young AdultABSTRACT
The objective of this study was to determine the effects of some minor components such as squalene and mono- and diglycerides (MDG) on the frying stability of olive pomace oil. Refined olive pomace oil was distilled using a falling film type short-path distillation unit at 230 °C under a pressure of 0.02 mbar to remove minor components. Distilled olive pomace oil was introduced with approximately 10,000 mg/kg of squalene and 2.5% of MDG. Fryings were performed 8 times/day at 180 °C for 3 min. Whole frying procedure proceeded for 5 days. Total polar compounds, polymerized triglycerides (PTG), free fatty acids, viscosity, color index, p-anisidine value, smoke point, fatty acid composition and iodine values of the oils were monitored. Results indicated that all criteria except smoke point and iodine value increased in all fractions during fryings. The lowest total polar compound was obtained in distilled olive pomace oil while PTG was the lowest in the MDG added fraction. Lower increases in viscosity and color indexes were recorded in squalene incorporated samples. It was observed that squalene and MDG were not effective on the oxidation rate of unsaturated fatty acids during frying.
ABSTRACT
Phytosterolemia is a rare autosomal recessive sterol storage disease caused by mutations in ABCG5 and ABCG8 genes. A 9-year-old Turkish boy who was presented with exclusively hematologic abnormalities had elevated plant sterol levels. Sequencing of ABCG5 and ABCG8 genes revealed a novel homozygous IVS10-1 G>T mutation in ABCG5 gene. Four of the 13 family members had xanthoma but they had neither hematologic abnormalities nor IVS10-1 G>T mutation. Ezetimibe therapy reduced plant sterol levels in association with marked clinical improvement. Plant sterol levels and ABCG5/ABCG8 genes should be analysed in patients with unexplained hemolytic anemia and macrothrombocytopenia.
